The significance of cystic kidney disease is determined by the following factors:
• Kidney cysts are very common and sometimes represent a complex diagnostic task for physicians-therapists, surgeons, radiologists and pathologists.
• Some forms, such as polycystic kidney disease in adults, are the main reason for including the patient in the hemodialysis program.
• Cysts can eventually turn into malignant tumors that are difficult to recognize.
Simple cysts
Simple cysts are single or multiple cystic formations of different sizes, from 1 to 10 cm in diameter. Cysts are usually surrounded by a smooth shiny grayish shell and filled with a clear liquid. In the kidney, cysts usually occupy a peripheral position (in the area of the cortex), although occasionally they may be located in the central part (in the medulla). Cysts in the kidneys, as a rule, do not cause any symptoms and are very common in people older than 50 years. With large sizes of cysts, pain in the lumbar region may occur, but such cysts are usually detected accidentally when examining the kidneys for another pathology. Cases of hemorrhage into the cysts are described, which the patient feels as sudden sharp pain in the side and lumbar region. Bleeding can be a herald of malignant degeneration of the cyst shell. With congenital multicystosis of the kidneys, the child is born with sharply increased non-functioning kidneys, the substance of which is turned into a multitude of cysts. With bilateral renal damage, the fetus is in utero unable to produce urine, resulting in much less amniotic fluid. This leads to deformations of the fetus due to increased pressure of the uterus. The face of such a fruit is skewed, the nose is flattened, the ears are located low, and deep folds under the eyes.
Kidney Removal
Patients with multicystic kidney are recommended nephrectomy - surgical removal of the kidney. The operation is performed in case of an increase or infection of the cysts, as well as with high blood pressure in the patient.
Polycystic is a genetically conditioned condition. There are several forms of the disease:
• perinatal - a child is born with huge kidneys and dies shortly after birth;
• nonsatal - is diagnosed during the first month of life;
• Children - in children aged 3 to 12 years, there is a developmental gap and kidney failure. Other signs include high blood pressure, enlargement of the liver and spleen;
• juvenile - the disease is detected during the first years of life;
• Adult - this condition develops in adult carriers of an autosomal dominant gene. This means that the sick individual inherited the disease gene from one of the parents.
The most common genetic defect in polycystic kidney disease in adults is a mutation in the 16th chromosome, which is responsible for the production of polycystin protein. The latter plays an important role in intercellular interactions. Symptoms of polycystic kidney include bloating, pain in the lumbar region, hematuria (blood in the urine) and high blood pressure. Kidney damage can be detected accidentally or as a result of examination of the relatives of the patient.
Diagnostics
In most patients, the disease is detected at the age of 30 to 50 years. A steady decrease in renal function is observed in about a third of patients and leads to the need for dialysis, and subsequently - kidney transplantation.
Concomitant symptoms
Polycystic can be accompanied by a number of other pathological symptoms, which include, in particular:
• hypertension (high blood pressure);
• Infection of renal cysts;
• aneurysms (bulging of the wall) of the brain and other arteries;
• Hernias and diverticula of the intestine.
Treatment
Patients on dialysis therapy or after transplantation may need to remove ever-increasing kidneys in order to prevent bleeding, infection and pain.
Other cystic kidney diseases include:
• Fanconi syndrome is a rare condition, inherited as an X-linked dominant trait. Characterized by anemia, kidney failure and low sodium in the blood.
• Spongy kidney - a sharp expansion of collecting tubes. A small part, whole or even both kidneys can be affected. This congenital or acquired condition is often combined with the tumor of Wilms (malignant kidney tumor in children), aniridia (absence of the iris of the eye) and hemyhypertrophy (hypertrophy of the muscles of one half of the body). The disease is often accompanied by recurrent urinary tract infections, stone formation and kidney failure.
• Hippel-Lindau disease is a serious family disease with the development of benign tumors of the cerebellum, retina, spine, sometimes the pancreas and adrenal gland in the context of cystic kidney damage, which tends to malignancy.
• Malignant cysts of the kidneys are the result of the destruction of the central part of the cancerous tumor with the formation of a cyst or, conversely, the malignancy of a benign cyst.